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Imaging Findings in a Case of Mycosis FungoidesNayha Handa,

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Imaging Findings in a Case of Mycosis Fungoides

Nayha Handa,1* MD, Navya Handa,2 MD, Venu Madhav,3 MD, Swapndeep Singh Atwal,3 MD, Puneet Garg,4 MD, UC Garga,3 MD

Address: 1Department of Radiodiagnosis, AIIMS, New Delhi, 2Department of Dermatology, Dr S.N. Medical College, Jodhpur, 3Department of Radiodiagnosis, PGIMER and Dr RML Hospital, New Delhi, 4Department of

Radiodiagnosis, SGPGI, Lucknow E-mail: nayhahanda@gmail.com

* Corresponding Author: Dr. Nayha Handa, Department of Radiodiagnosis, AIIMS, New Delhi

Case Report DOI: 10.6003/jtad.1591c4

Published:

J Turk Acad Dermatol 2015; 9 (1): 1591c4

This article is available from: http://www.jtad.org/2015/1/jtad1591c4.pdf Keywords: Mycosis fungoides, imaging findings

Abstract

Observation: Mycosis fungoides is a type of malignant T-cell lymphoma that primarily involves the skin. In its later stages, mycosis fungoides can cause peripheral lymphadenopathy and also progress to widespread extracutaneous visceral involvement. The cutaneous lesions including patches, plaques, or erythroderma show no abnormalities at CT. In the stage of tumor formation, thickening or a mass of the skin is seen at CT. Evaluation of extracutaneous involvement, disease progression, and stage is the most important role of imaging in mycosis fungoides. Extracutaneous involvement causes a dramatic decrease in the survival rate. Therefore, CT demonstration of clinically unsuspected lymphadenopathy or abnormality in visceral organs such as hepatosplenomegaly is important. We report the imaging findings of a patient of mycosis fungoides with extensive cutaneous, nodal and visceral and unusual thyroid gland involvement.

Introduction

Mycosis fungoides is a type of malignant T- cell lymphoma that primarily involves the skin. In its later stages, mycosis fungoides can cause peripheral lymphadenopathy and also progress to widespread extracutaneous visceral involvement.

Imaging plays a role in the evaluation of ext- racutaneous involvement, disease progres- sion, and staging mycosis fungoides.

Extracutaneous involvement causes a dra- matic decrease in the survival rate. Therefore, CT demonstration of clinically unsuspected lymphadenopathy or abnormality in visceral organs such as hepatosplenomegaly is impor- tant [1].

We report the imaging findings of a patient of mycosis fungoides with extensive cutaneous,

Page 1 of 3

(page number not for citation purposes) Figure 1. Patient showing multiple skin nodules

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nodal and visceral involvement and unusual involvement of the thyroid gland.

Case Report

A 46 year old male presented with itchy hypopig- mented patches which gradually progressed in size and number over the past two years. Patient also complained of nodular lesions all over the body (Figure 1), some of which show ulceration for the last two months.

Blood investigations showed Haemoglobin: 11 g/dl, RBC count:3.98 /mm3,TLC: 4000/mm3, Platelet count: 1.2 lakh/mm3, DLC: P78 L20 E02.

Peripheral smear showed normocytic normochro- mic anemia with anisocytosis and poikilocytosis.

Liver function tests, kidney function tests, serum electrolytes, thyroid function tests were within the normal range. HIV and VDRL tests were negative.

Ultrasound and Contrast enhanced CT of the pa- tient done in the Department of Radiology (Figure 2, 3 and 4).

Multiple isochoiec lesions with a hypoechoiec halo were noted in the thyroid on ultrasound with pe- ripheral vascularity on Colour Doppler (Figure 5).

These were seen as hypodense nonenhancing no- dules on CT. There was no calcification within these nodules.

CT revealed multiple nodules in lower lobes of bi- lateral lungs.

Multiple hypodense nonenhancing lesions were noted in the liver, pancreas and in bilateral kid- neys. A well defined lobulated lesion measuring 5x4.2 cm was noted in the left anterolateral abdo- minal wall. Similar smaller lesions noted in poste- rior abdominal wall. Multiple enhancing subcutaneous nodules noted.

Multiple lytic bone lesions associated with enhan- cing soft tissue component were seen involving the J Turk Acad Dermatol 2015; 9(1): 1591c4. http://www.jtad.org/2015/1/jtad1591c4.pdf

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(page number not for citation purposes) Figure 3. CECT showing hypodense lesions in the liver and pancreas. Expansile lytic soft tissue of the

right rib

Figure 4. CECT multiple enhancing subcuta- neous nodules

Figure 2. CT chest in lung window showing multiple nodules in bilateral lower lobes

Figure 5. Ultrasound of the thyroid showing isoechoiec lesions with halo

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scapula, right clavicle, posterior ends of right 9th, 10th and 11th ribs and left 7th rib and sacrum.

Biopsy from the subcutaneous lesions revealed pa- utriers abscesses and epidermotropic infiltrate of atypical lymphoid cells.

Discussion

Mycosis fungoides is a peripheral non-Hodg- kin's T-cell neoplastic process, representing the most common type of primary cutaneous malignant lymphoma [2]. The disorder is more common in males and in blacks. Mycosis fun- goides is an indolent lymphoma, with patients often having several years of eczematous skin lesions before the diagnosis is finally establis- hed. The cutaneous lesions including patches, plaques, or erythroderma show no abnormali- ties at CT. In the stage of tumor formation, thickening or a mass of the skin is seen at CT.

The imaging features of mycosis fungoides are nonspecific, and differentiation from cuta- neous involvement by other diseases including T-cell leukemia and from connective tissue di- sease is frequently not possible [1].

In its later stages, mycosis fungoides can cause peripheral lymphadenopathy and finally progress to widespread extracutaneous visce- ral involvement. Visceral involvement is a re- latively common but poorly appreciated. The lungs are the most common site of visceral in- volvement [3, 4, 5, 6]. The other usual sites of extracutaneous dissemination of mycosis fungoides are liver, spleen, and blood.

Thyroid involvement as seen in our case is ext- remely rare and has been reported in only a few case reports [4, 7]. Thyroid involvement is seen in the form of multiple nodules. Oral re- tinoids used in the treatment of MF decrease thyroxine production and thyroid hormone replacement might be required.

On histopathology small intraepidermal collec- tion of lymphocytes also called Pautrier mic- roabscess can be seen in early lesions. The normal structure of the skin is usually destro- yed in later stages [6]. The histological finding is a key due to lack of specific clinical finding or criteria.

Patients have been treated with radiation the- rapy, topical glucocorticoids, retinoids,topical nitrogen mustard, phototherapy, psoralen with ultraviolet A, electron beam radiation, in- terferon, and systemic cytotoxic therapy [8].

References

1. Lee HJ, Im JG, Goo JM, Kim WK, Choi BI, Han JK, et al. Peripheral T-Cell Lymphoma: Spectrum of ima- ging findings with clinical and pathologic features.

Radiographics 2003; 23: 7-26. PMID: 12533636 2. Howard MS, Smoller BR. Mycosis fungoides: classic

disease and variant presentations. Semin Cutan Med Surg 2000; 19: 91-99. PMID: 10892710

3. Kulin PA, Marglin SI, Shuman WP, Chew DE, Olerud JE. Diagnostic imaging in the initial staging of myco- sis fungoides and Sézary syndrome. Arch Dermatol 1990; 126: 914-918. PMID: 2360839

4. Gómez-De La Fuente E, Ortiz PL, Vanaclocha F, Rod- riguez-Peralto JL, Iglesias, L. Aggressive granuloma- tous mycosis fungoides with clinical pulmonary and thyroid involvement. Brit J Dermatol 2000; 142:

1026–1029. PMID: 10809867

5. Patel DJ, Griem ML, Vijayakumar S, Griem SF. Tre- atment of pulmonary mycosis fungoides with whole- lung radiation therapy. J Surg Oncol 1988; 38:

118-120. PMID: 3379966

6. Sander CA, Kind P, Kaudewitz P, Raffeld M, Jaffe ES.

The Revised European-American Classification of Lymphoid Neoplasms (REAL): a new perspective for the classification of cutaneous lymphomas. J Cutan Pathol 1997; 24: 329-341. PMID: 9243360

7. Balachandran S, Harper RR, Boyd CM. Lymphoma of the thyroid gland associated with mycosis fungoides:

sonographic, scintigraphic and CT features. Radiat Med 1984; 2: 211-213. PMID: 6399583

8. Maingon P, Truc G, Dalac S, Barillot I, Lambert D, Petrella T, et al. Radiotherapy of advanced mycosis fungoides: indications and results of total skin elect- ron beam and photon beam irradiation. Radiother Oncol 2000; 54: 73-78. PMID: 10719702

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(page number not for citation purposes) J Turk Acad Dermatol 2015; 9 (1): 1591c4. http://www.jtad.org/2015/1/jtad1591c4.pdf

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